小儿非横纹肌肉瘤类软组织肿瘤的临床特点及预后分析

Clinical characteristics and prognosis of pediatric non-rhabdomyosarcoma soft tissue sarcomas

目的分析小儿非横纹肌肉瘤类软组织肿瘤(NRSTS)的临床特点及预后,以提高对此类肿瘤临床特点及预后的认识。方法连续纳入2011年6月至2018年5月就诊于首都医科大学附属北京儿童医院血液肿瘤病房的NRSTS患儿共29例。重点收集患儿的临床及病理资料,分析其临床特点、国际横纹肌肉瘤协组(IRS)分期、小儿肿瘤协组(COG)危险度分组与预后关系。随访截止日期为2018年10月31日。采用Kaplan-Meier进行生存分析。结果共纳入29例患儿,男14例,女15例,年龄7~169个月,中位年龄为59.5个月。病理类型共10种,滑膜肉瘤、婴儿型纤维肉瘤、恶性横纹肌样瘤各5例,其他病理分型共14例。肿瘤原发于头颈部10例,四肢及躯干6例,内脏器官13例,发生远处转移者10例。IRS分期Ⅰ期及Ⅱ期患儿12例,Ⅲ期及Ⅳ期患儿17例。所有患儿均进行手术、化疗和/或放疗,术前化疗者9例,术后化疗者17例,无法手术切除者3例。中位随访时间16个月(4~89个月)。29例患儿死亡6例,其中恶性横纹肌样瘤最多,为3例。所有患儿2年总生存率为77.4%,2年无事件生存率为53.2%。单因素分析提示IRS分期、COG危险度分组及原发部位与预后相关。IRS分期Ⅰ~Ⅱ期与Ⅲ~Ⅳ期患儿2年无事件生存率分别为75.0%及35.9%(χ2=7.303,P=0.007),2年总生存率分别为100%及61.8%(χ2=4.81,P=0.028);COG危险度分组低危组与中高危组患儿2年无事件生存分别为66.7%及44.7%(χ2=4.155,P=0.042),2年总生存率分别为100%及66.3%(χ2=3.383,P=0.066);原发部位位于内脏及非内脏部位2年总生存率分别为59.3%及92.9%(χ2=4.202,P=0.04)。结论小儿NRSTS临床表现异质性强,治疗以手术治疗为主。原发部位位于内脏器官、IRS分期Ⅲ~Ⅳ患儿预后差。

Objective To analyze the clinical features and prognosis of pediatric non-rhabdomyosarcoma soft tissue sarcoma(NRSTS),and further understand these tumors.Methods Twenty-nine NRSTS children were admitted to the Hematological Oncology Ward of Beijing Children′s Hospital from June 2011 to May 2018.The clinical and pathological data of these children were collected,and the relationships of the prognosis with clinical characteristics Intergroup Rhabdomyosarcoma Study(IRS)stage,Children′s Oncology Group(COG)risk grouping were analyzed.All the patients were followed up until October 31,2018.The survival analysis was performed by Kaplan-Meier method.Results There were 14 boys and 15 girls in the enrolled 29 children,aging from 7 to 169 months,with a median age of 59.5 months.There were 10 pathological types,including synovial sarcoma,infantile fibrosarcoma and malignant rhabdomyoid tumors in 5 cases,and other pathological types in 14 cases.Tumors originated from the head and neck in 10 cases,limbs or trunk in 6 cases,visceral sites in 13 cases.Ten cases showed distant metastasis.There were 12 cases at IRS stageⅠandⅡ,and 17 cases of stageⅢandⅣ.All the children were treated with surgery and chemotherapy with or without radiotherapy.Nine cases received preoperative chemotherapy,17 cases received postoperative chemotherapy,3 cases could not be resected surgically.The follow-up period ranged from 4 to 89 months,with a median follow-up of 16 months.Six of 29 children died,including 3 cases of malignant rhabdomyoid sarcoma.The 2-year overall survival(OS)rate of all the children was 77.4%and the 2-year event-free survival(EFS)rate of all the children was 53.2%.By analyzing the clinical factors,it was found that the IRS stage,COG risk group,primary sites were related to prognosis.The 2-year EFS rate of children with IRSⅠ-ⅡandⅢ-Ⅳwere 75.0%and 35.9%,respectively(χ2=7.303,P=0.007),the 2-year OS rate was 100%and 61.8%,respectively(χ2=4.81,P=0.028);The 2-year EFS of children in COG low-risk group and median/high-risk group were 66.7%and 44.7%,respectively(χ2=4.155,P=0.042),the 2-year OS rate of children in COG low and median/high-risk was 100%and 66.3%,respectively(χ2=3.383,P=0.066);the 2-year OS rate of children in visceral and non-visceral sites were 59.3%and 92.9%,respectively(χ2=4.202,P=0.04).Conclusions NRSTS in children is heterogeneous,and surgery is the main treatment.Children with primary tumors located in visceral sites and at IRSⅢ-Ⅳhad poor outcomes.